Hypoplasia of the cerebellar vermis and corpus callosum in thrombocytopenia with absent radius syndrome on MRI studies

Thrombocytopenia with absent radius (TAR) syndrome is infrequently (7%) associated with mental retardation. In those cases, the mental deficiency is presumed to be a consequence of intracranial hemorrhage due to the thrombo-cytopenia. We report on 2 infants with TAR syndrome. One had developmental delay with evidence of cerebral dysgenesis by magnetic resonance imaging (MRI). Such findings have not been noted in the literature, but may not have been investigated in most cases. The other infant with TAR syndrome, who has had normal psychomotor development, has a normal brain on MRI scan. Detailed neuroimaging studies, preferably MRI, should be considered in the evaluation of patients with TAR syndrome, especially when there are documented signs of developmental delay, with or without a history of intracranial hemorrhage. © 1994 Wiley-Liss, Inc.     

Evaluation of Questor urine screening system for bacteriuria and pyuria.

AIMS--To evaluate the Questor automated bacteriuria and pyuria screening system; to compare its performance with that of a reference method; and to assess its usefulness in a routine clinical laboratory. METHODS--The Questor urine screening system was compared with a comprehensive regimen to detect urinary tract infection, using pour-plate viable counts to determine the numbers of bacteria present in urine samples, a wide range of other cultural methods, microscopic findings and clinical information. RESULTS--The optimal performance in detecting significant growths was a sensitivity of 93%, a specificity of 74%, a positive predictive value of 43% and a negative predictive value of 98%. The list price per test is 0.17 pounds and the capital cost of the system is 39,950 pounds. Questor can test 50 samples an hour and can be operated by one member of the laboratory staff, who is not required to make interpretative judgments--for example, a medical laboratory assistant. CONCLUSIONS--The sensitivity and specificity of the Questor was better than that obtained from other screening systems using the same protocol. The system was easy to use and is a useful addition to the methods available for screening for bacteriuria.     

Expression of the CD28 costimulatory molecule on subsets of murine intestinal intraepithelial lymphocytes correlates with lineage and responsiveness

The CD28 antigen has been recently demonstrated to be a costimulatory molecule and is expressed by almost all thymic and peripheral T cell receptor (TcR) α^δ+ and γ^λ+ cells in the mouse system. We show here that expression of CD28 is heterogeneous among murine intestinal intraepithelial lymphocytes (IEL). Whereas some TcR αβ-expressing IEL subsets such as CD4⁺8^? and CD4^?8α⁺β⁺ cells express CD28 at the same levels as their phenotypic counterparts in lymph node, other subsets of TcR αβ cells (including CD4^?8α⁺β^? and CD4⁺8αβ⁺β cells) as well as TcR γ^λ+ IEL fail to express CD28. Parallel experiments using aged BALB/c-nu/nu mice indicated that CD28 expression patterns among IEL are quite similar to those of normal BALB/c mice. Furthermore, forward light scatter analysis showed that CD28^? cells are considerably larger than CD28⁺ cells in the gut, although cycling cells were rare in both subsets. Finally CD28^? cells in the gut did not proliferate or produce IL-2 upon stimulation by anti-CD3 monoclonal antibodies (mAb) and phorbol 12-myristate 13-acetate, whereas CD28⁺ cells in the gut and lymph nodes responded to these stimuli. The response of the CD28⁺ cells was enhanced by anti-CD28 mAb. These results suggest that CD28^? IEL (CD4-8α⁺β^? cells, and some CD4⁺8α⁺β^?cells) may follow a different developmental pathway from that of CD28⁺ IEL in a thymus-independent environment, and that expression of CD28 correlates with responsiveness of the cells to triggering via the TcR-CD3 complex.     

Italian version of the organic brain syndrome and the depression scales from the CARE: evaluation of their performance in geriatric institutions

The Organic Brain Syndrome (OBS) and the Depression (D) scales derived from the Comprehensive Assessment and Referral Evaluation (CARE) were translated into Italian and used in a survey of geriatric institutions in Milan. During the survey validity and reliability tests of the scales were conducted. Inter-rater reliability (total score weighted kappa) was highly satisfactory for both scales (0.96 for OBS and 0.83 for D scale). Reliability was assessed three times during the survey and showed good stability for both scales, with a slight but significant trend towards reduction over time for the D scale. Reliability of the D scale was significantly lower when the subjects interviewed scored highly on the OBS scale (severe cognitive impairment). Criterion validity was highly satisfactory both for the OBS scale (cut-off point 4/5: sensitivity 77%, specificity 96%, positive predictive value 91%) and the D scale (cut-off point 10/11: sensitivity 95%, specificity 92%, positive predictive value 84%). Results are discussed with special reference to longitudinal assessment of reliability, the choice of the cut-off point, and the context-dependent properties of questionnaires.     

A CD3- subset of CD4-8+ thymocytes: a rapidly cycling intermediate in the generation of CD4+8+ cells

T lymphocytes with the surface phenotype CD4+8- and CD4-8+ are considered to be representative of functionally mature cells. We show here that adult murine thymus contains a subpopulation of CD4-8+ cells that differ from CD4-8+ cells found in the periphery in that they do not express the T cell receptor-associated CD3 molecular complex. Such CD3-4-8+ thymocytes are cortisone sensitive and rapidly cycling in situ. Furthermore, in contrast to mature T cells, most CD3-4-8+ thymocytes express low levels of CD5 and high levels of the B2A2 antigen. CD3-4-8+ thymocytes fail to respond to a variety of mitogenic stimuli in vitro but do give rise upon short-term culture to CD4+8+ cells. It is suggested that CD3-4-8+ thymocytes represent a transitional stage of thymus differentiation between the CD4-8- and CD4+8+ compartments.     

Skewed T cell receptor V{alpha} repertoire among superantigen reactive murine T cells

Reactivity of murine T cells with viral or bacterial superantigensis clearly correlated with the expression of TCR V_ßdomains. Thus, T cells responding to the minor lymphocyte stimulatorylocus (Mls-1^a) or staphylococcal enterotoxin B (SEB) expresspredominantly TCR V_ß6 or V_ß8.2 respectively.We have investigated the involvement of the other major variableelement of the TCR, the V domain, in these superantigen responses.Using a panel of anti-TCR V mAbs, It is demonstrated that theTCR V repertoire among superantigen stimulated V_ß6⁺or V_ß8.2⁺ blasts (responding to Mls-1^a or SEB respectivelyin vitro) is altered in comparison with anti-CD3 stimulatedcells expressing the same V domains. Furthermore, the TCR Vrepertoire is strongly skewed in TCR V_ß8.2 transgenicmice that have undergone extensive peripheral clonal deletionafter SEB injection. These data imply that the V domain influencessuperantigen recognition by sthe TCR.